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Cardiology280 papers

Systemic rheumatoid vasculitis

Last edited: 4/23/2026

Overview

Systemic rheumatoid vasculitis is a rare, heterogeneous group of inflammatory disorders characterized by vascular inflammation affecting multiple organs, leading to significant morbidity and mortality 123.

Diagnosis

  • Clinical Presentation: Multi-organ involvement, often with constitutional symptoms like fever and weight loss 17.
  • Laboratory Tests: Elevated inflammatory markers, presence of ANCA (anti-neutrophil cytoplasmic antibodies) 1141.
  • Imaging: MRI, CT, and angiography to assess vascular involvement and organ damage 221.
  • Histopathology: Biopsy showing characteristic vascular inflammation and necrosis 50.
  • Serological Markers: Anti-endothelial cell autoantibodies (AECA) may be present in ANCA-associated vasculitis 51.
  • Classification Algorithms: Use of consensus methodology for accurate classification 43.
  • Management

  • First-Line Treatments: Corticosteroids (e.g., prednisone) combined with immunosuppressive agents like cyclophosphamide 41956.
  • Adjunctive Therapies: Rituximab for refractory cases 3255.
  • Biological Agents: TNF-α inhibitors (e.g., infliximab) for specific manifestations or refractory disease 6071.
  • Monitoring: Regular assessment using tools like the Birmingham Vasculitis Activity Score (BVAS) 4852.
  • Cardiovascular Monitoring: Given the risk of cardiovascular involvement, regular echocardiograms and MRI may be necessary 42.
  • Management of Comorbidities: Addressing smoking cessation due to its association with organ damage 77.
  • Special Populations

  • Pregnancy: High-risk pregnancies; outcomes vary; close monitoring and individualized management required 11246.
  • Pediatrics: Unique challenges; tailored immunosuppressive strategies and multidisciplinary care essential 101433.
  • Elderly: Increased risk of complications; careful titration of immunosuppressive therapy to balance efficacy and safety 7.
  • Comorbidities: Consider impact of existing conditions like hepatitis C virus infection on treatment and prognosis 49.
  • Key Recommendations

  • Initiate corticosteroids and cyclophosphamide for severe systemic vasculitis (Evidence: Strong 4).
  • Use BVAS for regular assessment of disease activity (Evidence: Moderate 4852).
  • Consider rituximab in patients refractory to conventional therapy (Evidence: Moderate 3255).
  • Monitor cardiovascular health closely due to increased risk of involvement (Evidence: Moderate 42).
  • Tailor management in pregnant women with systemic vasculitis to optimize maternal and fetal outcomes (Evidence: Moderate 11246).
  • References

    Showing 100 most recent of 264 indexed papers.

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Diagnostic delays in systemic vasculitides. Rheumatology international 2024. link 8 Mirouse A, Cacoub P, Saadoun D. Regulatory T cells and systemic vasculitis. Current opinion in rheumatology 2023. link 9 Tsuji K, Okuyama Y, Asano Y, Yamaoka K, Kitamura S, Wada J. Co-occurrence of Three Systemic Diseases: ANCA-associated Vasculitis, Sjögren's syndrome and Sarcoidosis. Internal medicine (Tokyo, Japan) 2023. link 10 Sener S, Arslanoglu Aydin E, Batu ED. Cardiac involvement and cardiovascular risk factors in pediatric primary systemic vasculitides. Clinical rheumatology 2023. link 11 Conticini E, d'Alessandro M, Al Khayyat SG, D'Alessandro R, D'Ignazio E, Pata AP et al.. Inflammatory muscle involvement in systemic vasculitis: A systematic review. Autoimmunity reviews 2022. link 12 Nguyen V, Wuebbolt D, Pagnoux C, D'Souza R. Pregnancy outcomes in women with primary systemic vasculitis: a retrospective study. The journal of maternal-fetal & neonatal medicine : the official journal of the European Association of Perinatal Medicine, the Federation of Asia and Oceania Perinatal Societies, the International Society of Perinatal Obstetricians 2021. link 13 Gibson KM, Kain R, Luqmani RA, Ross CJ, Cabral DA, Brown KL. Autoantibodies Against Lysosome Associated Membrane Protein-2 (LAMP-2) in Pediatric Chronic Primary Systemic Vasculitis. Frontiers in immunology 2020. link 14 de Graeff N, Groot N, Brogan P, Ozen S, Avcin T, Bader-Meunier B et al.. European consensus-based recommendations for the diagnosis and treatment of rare paediatric vasculitides - the SHARE initiative. Rheumatology (Oxford, England) 2019. link 15 Watts RA. Evolving concepts in classification of systemic vasculitis: where are we and what is the way forward?. International journal of rheumatic diseases 2019. link 16 Brogan P, Eleftheriou D. Vasculitis update: pathogenesis and biomarkers. Pediatric nephrology (Berlin, Germany) 2018. link 17 Thorpe CT, Thorpe JM, Jiang T, Atkinson D, Kang Y, Schleiden LJ et al.. Healthcare utilization and expenditures for United States Medicare beneficiaries with systemic vasculitis. Seminars in arthritis and rheumatism 2018. link 18 Nakano N, Mori M, Umebayashi H, Iwata N, Kobayashi N, Masunaga K et al.. Characteristics and outcome of intractable vasculitis syndrome in children: Nation-wide survey in Japan. Modern rheumatology 2018. link 19 Moussi-Frances J, Sallée M, Jourde-Chiche N. Apheresis to treat systemic vasculitis. Joint bone spine 2018. link 20 Soowamber M, Weizman AV, Pagnoux C. Gastrointestinal aspects of vasculitides. Nature reviews. Gastroenterology & hepatology 2017. link 21 Khosla A, Andring B, Atchie B, Zerr J, White B, MacFarlane J et al.. Systemic Vasculopathies: Imaging and Management. Radiologic clinics of North America 2016. link 22 Moreno-Ariño M, Ortiz-Santamaria V, Deudero Infante A, Ayats Delgado M, Novell Teixidó F. A classic mimicker of systemic vasculitis. Reumatologia clinica 2016. link 23 Lioger B, Diot E, Ebbo M, Schleinitz N, Aaron L, Michot JM et al.. Posterior reversible encephalopathy syndrome and systemic vasculitis: report of six cases. Clinical and experimental rheumatology 2016. link 24 Misra DP, Agarwal V. Innate immune cells in the pathogenesis of primary systemic vasculitis. Rheumatology international 2016. link 25 Sawalha AH, Dozmorov MG. Epigenomic functional characterization of genetic susceptibility variants in systemic vasculitis. Journal of autoimmunity 2016. link 26 Khan H, Chaubey S, Nitiahpapand R, Desai J. A Rare Case of Florid Antineutrophil Cytoplasmic Autoantibody-Associated Vasculitis Involving Both Atrioventricular Valves. The Annals of thoracic surgery 2016. link 27 Kim DS, Shin D, Kim TG, Kim SH, Kim DY, Kim SM et al.. Red blood cell distribution width as a useful indicator to predict systemic vasculitis in patients with cutaneous vasculitis. Rheumatology international 2015. link 28 Romero-Gómez C, Aguilar-García JA, García-de-Lucas MD, Cotos-Canca R, Olalla-Sierra J, García-Alegría JJ et al.. Epidemiological study of primary systemic vasculitides among adults in southern Spain and review of the main epidemiological studies. Clinical and experimental rheumatology 2015. link 29 Sharma A, Mittal T, Rajan R, Rathi M, Nada R, Minz RW et al.. Validation of the consensus methodology algorithm for the classification of systemic necrotizing vasculitis in Indian patients. International journal of rheumatic diseases 2014. link 30 Lega JC, Seror R, Fassier T, Aumaître O, Quere I, Pourrat J et al.. Characteristics, prognosis, and outcomes of cutaneous ischemia and gangrene in systemic necrotizing vasculitides: a retrospective multicenter study. Seminars in arthritis and rheumatism 2014. link 31 Mohammad A, Segelmark M. Primary systemic vasculitis with severe α1-antitrypsin deficiency revisited. Scandinavian journal of rheumatology 2014. link 32 Silva-Fernández L, Loza E, Martínez-Taboada VM, Blanco R, Rúa-Figueroa I, Pego-Reigosa JM et al.. Biological therapy for systemic vasculitis: a systematic review. Seminars in arthritis and rheumatism 2014. link 33 Sharma A, Gopalakrishan D, Nada R, Kumar S, Dogra S, Aggarwal MM et al.. Uncommon presentations of primary systemic necrotizing vasculitides: the Great Masquerades. International journal of rheumatic diseases 2014. link 34 Lenert P, Icardi M, Dahmoush L. ANA (+) ANCA (+) systemic vasculitis associated with the use of minocycline: case-based review. Clinical rheumatology 2013. link 35 Puéchal X, Guillevin L. Therapeutic immunomodulation in systemic vasculitis: taking stock. Joint bone spine 2013. link 36 Rothschild PR, Pagnoux C, Seror R, Brézin AP, Delair E, Guillevin L. Ophthalmologic manifestations of systemic necrotizing vasculitides at diagnosis: a retrospective study of 1286 patients and review of the literature. Seminars in arthritis and rheumatism 2013. link 37 Kallenberg CG. Could we abandon cyclophosphamide in systemic vasculitis and lupus nephritis?. Annals of the rheumatic diseases 2013. link 38 Flores-Suárez LF. Vasculitis: report from Mexico. Clinical and experimental nephrology 2013. link 39 Thurner L, Preuss KD, Fadle N, Regitz E, Klemm P, Zaks M et al.. Progranulin antibodies in autoimmune diseases. Journal of autoimmunity 2013. link 40 Wolf J, Schmitt V, Palm F, Grau AJ, Bergner R. Peripheral neuropathy as initial manifestation of primary systemic vasculitides. Journal of neurology 2013. link 41 Radice A, Bianchi L, Sinico RA. Anti-neutrophil cytoplasmic autoantibodies: methodological aspects and clinical significance in systemic vasculitis. Autoimmunity reviews 2013. link 42 Mavrogeni S, Cantini F, Pohost GM. Systemic vasculitis: an underestimated cause of heart failure - assessment by cardiovascular magnetic resonance. Reviews in cardiovascular medicine 2013. link 43 Kamali S, Artim-Esen B, Erer B, Ozdener L, Gul A, Ocal L et al.. Re-evaluation of 129 patients with systemic necrotizing vasculitides by using classification algorithm according to consensus methodology. Clinical rheumatology 2012. link 44 Xu PC, Chen M, Zhao MH. High potential to reverse the inhibition of myeloperoxidase by ceruloplasmin of anti-myeloperoxidase autoantibodies of IgG3 subclass. Autoimmunity 2012. link 45 Parra-García GD, Callejas-Rubio JL, Ríos-Fernández R, Sainz-Quevedo M, Ortego-Centeno N. Otolaryngologic manifestations of systemic vasculitis. Acta otorrinolaringologica espanola 2012. link 46 Gatto M, Iaccarino L, Canova M, Zen M, Nalotto L, Ramonda R et al.. Pregnancy and vasculitis: a systematic review of the literature. Autoimmunity reviews 2012. link 47 Mohammad AJ, Segelmark M. Association of cigarette smoking with organ damage in primary systemic vasculitis. Scandinavian journal of rheumatology 2011. link 48 Suppiah R, Mukhtyar C, Flossmann O, Alberici F, Baslund B, Batra R et al.. A cross-sectional study of the Birmingham Vasculitis Activity Score version 3 in systemic vasculitis. Rheumatology (Oxford, England) 2011. link 49 Terrier B, Sène D, Dechartres A, Saadoun D, Ortonne N, Rouvier P et al.. Systemic vasculitis in patients with hepatitis C virus infection with and without detectable mixed cryoglobulinemia. The Journal of rheumatology 2011. link 50 Hervier B, Durant C, Masseau A, Ponge T, Hamidou M, Mussini JM. Use of muscle biopsies for diagnosis of systemic vasculitides. The Journal of rheumatology 2011. link 51 Hu N, Westra J, Huitema MG, Stegeman CA, Limburg PC, Kallenberg CG. Autoantibodies against glomerular endothelial cells in anti-neutrophil cytoplasmic autoantibody-associated systemic vasculitis. Nephrology (Carlton, Vic.) 2009. link 52 Mukhtyar C, Lee R, Brown D, Carruthers D, Dasgupta B, Dubey S et al.. Modification and validation of the Birmingham Vasculitis Activity Score (version 3). Annals of the rheumatic diseases 2009. link 53 Gedalia A, Cuchacovich R. Systemic vasculitis in childhood. Current rheumatology reports 2009. link 54 Mukhtyar C, Brogan P, Luqmani R. Cardiovascular involvement in primary systemic vasculitis. Best practice & research. Clinical rheumatology 2009. link 55 Phillip R, Luqmani R. Mortality in systemic vasculitis: a systematic review. Clinical and experimental rheumatology 2008. link 56 Statkute L, Oyama Y, Barr WG, Sufit R, Ho S, Verda L et al.. Autologous non-myeloablative haematopoietic stem cell transplantation for refractory systemic vasculitis. Annals of the rheumatic diseases 2008. link 57 Silveira LH. Damage assessment in systemic vasculitis. Current rheumatology reports 2008. link 58 Danila MI, Bridges SL. Update on pathogenic mechanisms of systemic necrotizing vasculitis. Current rheumatology reports 2008. link 59 Fiehn C, Ho AD, Lorenz HM. Hematopoietic stem cell transplantation (HSCT) for primary systemic vasculitis and related diseases. Autoimmunity 2008. link 60 Ashok D, Dubey S, Tomlinson I. C-ANCA positive systemic vasculitis in a patient with rheumatoid arthritis treated with infliximab. Clinical rheumatology 2008. link 61 Wikman A, Lundahl J, Jacobson SH. Sustained monocyte activation in clinical remission of systemic vasculitis. Inflammation 2008. link 62 Gao Y, Chen M, Ye H, Guo XH, Zhao MH, Wang HY. The target antigens of antineutrophil cytoplasmic antibodies (ANCA) induced by propylthiouracil. International immunopharmacology 2007. link 63 Serna-Candel C, Moreno-Perez O, Soriano V, Martínez A. Churg-Strauss syndrome triggered by hyposensitization to Alternaria fungus. Clinical rheumatology 2007. link 64 Pamuk GE, Uyanik MS, Demir M, Tekgündüz E, Turgut B, Soy M. Systemic antineutrophil cytoplasmic antibody vasculitis in a patient with chronic lymphocytic leukemia: quite a rare diagnosis. Leukemia research 2007. link 65 Quéméneur T, Martin-Nizard F, Kandoussi A, Kyndt X, Vanhille P, Hachulla E et al.. PON1, a new biomarker of cardiovascular disease, is low in patients with systemic vasculitis. Seminars in arthritis and rheumatism 2007. link 66 Abdulwahab A, Almoallim H, Khan N. Isolated eosinophilic mesenteric vasculitis with extensive thrombosis and splenic infarction in a 13-year-old boy. Clinical rheumatology 2007. link 67 Flossmann O, Bacon P, de Groot K, Jayne D, Rasmussen N, Seo P et al.. Development of comprehensive disease assessment in systemic vasculitis. Annals of the rheumatic diseases 2007. link 68 Mukhtyar C, Hellmich B, Jayne D, Flossmann O, Luqmani R. Remission in antineutrophil cytoplasmic antibody-associated systemic vasculitis. Clinical and experimental rheumatology 2006. link 69 Little MA, Bhangal G, Smyth CL, Nakada MT, Cook HT, Nourshargh S et al.. Therapeutic effect of anti-TNF-alpha antibodies in an experimental model of anti-neutrophil cytoplasm antibody-associated systemic vasculitis. Journal of the American Society of Nephrology : JASN 2006. link 70 Koukoulaki M, Jayne DR. Mycophenolate mofetil in anti-neutrophil cytoplasm antibodies-associated systemic vasculitis. Nephron. Clinical practice 2006. link 71 Raza K, Carruthers DM, Stevens R, Filer AD, Townend JN, Bacon PA. Infliximab leads to a rapid but transient improvement in endothelial function in patients with primary systemic vasculitis. Annals of the rheumatic diseases 2006. link 72 de Kort SW, van Rossum MA, ten Cate R. Infliximab in a child with therapy-resistant systemic vasculitis. Clinical rheumatology 2006. link 73 Miranda-Filloy JA, Veiga JA, Juarez Y, Gonzalez-Juanatey C, Gonzalez-Gay MA, Garcia-Porrua C. Microscopic polyangiitis following recurrent Staphylococcus aureus bacteremia and infectious endocarditis. Clinical and experimental rheumatology 2006. link 74 Kamali S, Inanc M, Gul A, Ocal L, Polat NG, Kilicaslan I et al.. Systemic necrotizing vasculitides in Turkey: a comparative analysis of 40 consecutive patients. Rheumatology international 2005. link 75 Kötter I, Daikeler T, Amberger C, Tyndall A, Kanz L. Autologous stem cell transplantation of treatment-resistant systemic vasculitis--a single center experience and review of the literature. Clinical nephrology 2005. link 76 Chen M, Yu F, Zhang Y, Zhao MH. Clinical [corrected] and pathological characteristics of Chinese patients with antineutrophil cytoplasmic autoantibody associated systemic vasculitides: a study of 426 patients from a single centre. Postgraduate medical journal 2005. link 77 Lode HM, Schmidt-Ioanas M. Vasculitis and infection: effects of immunosuppressive therapy. Clinical nephrology 2005. link 78 Viemann D, Strey A, Janning A, Jurk K, Klimmek K, Vogl T et al.. Myeloid-related proteins 8 and 14 induce a specific inflammatory response in human microvascular endothelial cells. Blood 2005. link 79 Hermann J, Demel U, Stünzner D, Daghofer E, Tilz G, Graninger W. Clinical interpretation of antineutrophil cytoplasmic antibodies: parvovirus B19 infection as a pitfall. Annals of the rheumatic diseases 2005. link 80 Brautbar N, Richter ED, Nesher G. Systemic vasculitis and prior recent exposure to organic solvents: report of two cases. Archives of environmental health 2004. link 81 Jayne D. The clinical features and pathology of vasculitis associated with anti-myeloperoxidase autoantibodies. Japanese journal of infectious diseases 2004. link 82 Williams J, Hewins P, Savage C. The influence of autoantibodies to myeloperoxidase on neutrophil function and intracellular signaling. 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Prognosis and outcome of 26 patients with systemic necrotizing vasculitis admitted to the intensive care unit. Rheumatology (Oxford, England) 2003. link 88 Rarok AA, Huitema MG, van der Leij MJ, van der Geld YM, Berthold H, Schmitt J et al.. Recombinant protein to analyze autoantibodies to proteinase 3 in systemic vasculitis. American journal of clinical pathology 2003. link 89 Koutantji M, Harrold E, Lane SE, Pearce S, Watts RA, Scott DG. Investigation of quality of life, mood, pain, disability, and disease status in primary systemic vasculitis. Arthritis and rheumatism 2003. link 90 Mulloy KB. Silica exposure and systemic vasculitis. Environmental health perspectives 2003. link 91 Bartolucci P, Ramanoelina J, Cohen P, Mahr A, Godmer P, Le Hello C et al.. Efficacy of the anti-TNF-alpha antibody infliximab against refractory systemic vasculitides: an open pilot study on 10 patients. Rheumatology (Oxford, England) 2002. link 92 Savage CO. The evolving pathogenesis of systemic vasculitis. Clinical medicine (London, England) 2002. link 93 Savige J, Nassis L, Cooper T, Paspaliaris B, Martinello P, MacGregor D. Antineutrophil cytoplasmic antibody (ANCA)-associated systemic vasculitis after immunisation with bacterial proteins. Clinical and experimental rheumatology 2002. link 94 McLaren JS, McRorie ER, Luqmani RA. Diagnosis and assessment of systemic vasculitis. Clinical and experimental rheumatology 2002. link 95 Lane SE, Watts RA, Barker TH, Scott DG. Evaluation of the Sørensen diagnostic criteria in the classification of systemic vasculitis. Rheumatology (Oxford, England) 2002. link 96 Poomthavorn P, Mahachoklertwattana P, Tapaneya-Olarn W, Chuansumrit A, Chunharas A. Antineutrophilic cytoplasmic antibody-positive systemic vasculitis associated with propylthiouracil therapy: report of 2 children with Graves' disease. Journal of the Medical Association of Thailand = Chotmaihet thangphaet 2002. link 97 Day C, Savage C. Primary systemic vasculitis. Minerva medica 2001. link 98 Garcia de La Peña Lefebvre P, Mouthon L, Cohen P, Lhote F, Guillevin L. Polyarteritis nodosa and mixed cryoglobulinaemia related to hepatitis B and C virus coinfection. Annals of the rheumatic diseases 2001. link 99 McLaren JS, Stimson RH, McRorie ER, Coia JE, Luqmani RA. The diagnostic value of anti-neutrophil cytoplasmic antibody testing in a routine clinical setting. QJM : monthly journal of the Association of Physicians 2001. link 100 Ben-Smith A, Dove SK, Martin A, Wakelam MJ, Savage CO. Antineutrophil cytoplasm autoantibodies from patients with systemic vasculitis activate neutrophils through distinct signaling cascades: comparison with conventional Fcgamma receptor ligation. Blood 2001. link

    Original source

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